VEXAS Syndrome with an Improvement of Chest Shadows without Immunosuppressive Therapy.
Kazuki Tanaka, Akira Kawamura, Ryo Suzuki et al.
✦ AI-curated · Sources linked
Case in brief
An 86-year-old man presented with elevated C-reactive protein, bicytopenia, and bilateral chest shadows. Bronchoalveolar lavage showed an increased lymphocyte count. Bone marrow vacuoles and a UBA1 c.122T>C (p.Met41Thr) variant with a high variant allele frequency established VEXAS syndrome. He declined treatment; the chest shadows improved without immunosuppressive therapy and remained stable for two months, even as systemic inflammatory symptoms and elevated C-reactive protein persisted.
What made this case unusual
Pulmonary shadows improved without immunosuppressive therapy despite a high UBA1 variant allele frequency and lymphocyte-predominant lavage, while systemic inflammation continued.
Diagnostic / clinical pearl
In suspected VEXAS, bone marrow vacuoles and a pathogenic UBA1 variant can support diagnosis; pulmonary findings may improve independently of systemic inflammatory activity.
Why it matters
The discordant course in this patient underscores the clinical heterogeneity of VEXAS and the need to consider the timing of treatment.